子宫浆液性乳头状癌24例临床病理分析

目的比较子宫浆液性乳头状癌(uterine papillary serous carcinoma,UPSC)和子宫内膜样癌(uterine endometrioid carcino-ma,UEC)的组织病理学和免疫组化表达,以了解两种肿瘤生物学行为的差异。方法对24例UPSC和54例UEC进行组织学复查和应用免疫组化SP法检测肿瘤的p53蛋白、ER和PR的表达。结果24例UPSC占子宫内膜癌的3·77%,平均年龄UP-SC组为60岁,UEC组为51·7岁(P<0·01),晚期癌(FIGOⅢ-Ⅳ)UPSC组占62·5%,UEC组占35·1%(P<0·025)。p53蛋白的表达UPSC组16例阳性(66·7%),UEC组10例阳性(18·5%),两组比较(P<0·01)。ER阳性表达UPSC组(8·3%),UEC组(42·5%),PR阳性表达UPSC组(12·5%),UEC组(35·2%),两组比较:ER(P<0·01),PR(P<0·05),差异有显著性。UPSC组p53蛋白表达与肿瘤分期、分级、及肌层浸润无明显关系,而UEC组Ⅲ~Ⅳ期肿瘤的p53蛋白的表达率高于Ⅰ-Ⅱ期(P<0·005)。UPSC的5年生存率为25%,UEC组5年生存率为80·9%(P<0.01),两组差异有显著性。结论UPSC为p53高表达,而缺乏雌激素和孕激素受体,为高度恶性的肿瘤。它的生物学行为不同于UEC,因而强调诊断时需和其他类型的子宫内膜癌相区别。     

Primary peritoneal serous carcinoma: a clinicopathological and immunohistochemical study of six cases

Aims: Primary peritoneal serous carcinoma (PPSC) is an unusual neoplasm that has not been properly characterized. To better define the clinicopathological and immunohistochemical features of PPSC, we present 6 such cases. Methods: The 6 patients consisted of one man and 5 women, ranging in age from 45 and 75 years. None of the patients had any history or clinical evidence of tumor elsewhere. The immunohistochemical profile was examined using antibodies against β-catenin, E-cadherin, wnt5a, EGFR, VEGF, vimentin, Ki67, and P53. Results: Of all the 6 PPSC cases, 5 cases presented stage IIIC and 1 case presented stage IV. Microscopically, 5 cases were poorly differentiated and 1 was moderately differentiated. All cases showed positive staining for β-catenin, E-cadherin, vimentin, VEGF, P53, and Ki67, 4 cases expressed EGFR, and all cases were consistently negative for wnt5a. Conclusions: We described 6 cases of PPSC with clinicopathological and immunohistochemical features. The findings provide basic knowledge of PPSC.     

Minimal uterine serous carcinoma: current concepts in diagnosis and prognosis

Uterine serous carcinoma (USC) is an aggressive type of endometrial cancer with a propensity to have extra-uterine spread at diagnosis, in some cases despite limited involvement of the uterus. Serous endometrial intra-epithelial carcinoma (EIC) is a recently recognised entity with the same cytological features and p53 mutations as USC, but it does not demonstrate stromal or myometrial invasion. In addition to representing the putative precursor to USC, the pure form of serous EIC may also be associated with extra-uterine tumour at the time of diagnosis and with risk for recurrence, spread, and eventual death from tumour. Current evidence indicates that serous EIC is a form of minimal USC with behaviour that is stage dependent, thereby necessitating complete surgical staging despite limited disease in the uterus. We review the diagnostic criteria for minimal USC, pitfalls in the differential diagnosis, and discuss a practical approach to evaluating biopsies, polypectomies, or hysterectomies containing minimal USC.     

Esthesioneuroblastoma: a Danish clinicopathological study of 40 consecutive cases

Aims. To review all cases of esthesioneuroblastoma in Denmark from 1978 to 2000 with respect to staging, grading, histopathological and immunohistochemical evaluation, and prognostication. Methods and results. Possible cases of esthesioneuroblastoma were retrieved from Danish oncology departments. Patients were included on the basis of review of their files or pathology reports, and/or on the basis of histopathological and immunohistochemical examination. Forty-nine possible cases were retrieved. Nine cases were excluded. Esthesioneuroblastoma is a malignant neuroendocrine tumour originating in the olfactory mucosa. It is a small blue cell neoplasm with a characteristic lobular architecture. It has a neuroendocrine immunophenotype and a sustentacular S-100 staining pattern. The tumours were staged according to Kadish and graded according to Hyams. Kaplan-Meier survival analysis was used to identify prognostic factors. Conclusion. The Kadish staging system was able to group the patients into prognostically relevant groups. Intracranial involvement and metastases at the time of diagnosis were found to be poor prognostic factors. Hyams grading system is difficult to work with and it was not possible to divide patients into prognostically relevant groups. Presence of necrosis, a diffuse growth pattern and a high proliferation index proved to be equally poor prognostic factors.     

Pseudoangiosarcomatous carcinoma: a clinicopathological study of seven cases

Seven cases of carcinoma mimicking angiosarcoma occurring in skin (3 cases), breast (3) and lung (1) are described. The cutaneous, pulmonary and one of the breast carcinomas were poorly differentiated and squamous in type; the other two breast tumours were poorly differentiated ductal carcinomas with focal squamous differentiation. Histologically, the pseudoangiosarcomatous pattern was due to complex anastomosing channels and spaces lined by neoplastic cells. The spaces contained hyaluronic acid. The neoplastic cells exhibited cytokeratin positivity but yielded negative results with the endothelial cell markers, factor VIII-related antigen and CD 34 (QB-END/10). Two breast tumours showed binding of UEA-1. Ultrastructurally, unequivocal epithelial differentiation was demonstrated in six of the cases. Pathogenetically, these tumours appeared to be variants of acantholytic squamous cell carcinoma. Recognition of this unusual form of carcinoma is important, as an incorrect diagnosis of angiosarcoma may lead to inappropriate treatment and prognostication.     

腹膜浆液性乳头状腺癌4例并临床病理分析

目的探讨腹膜浆液性乳头状腺癌(peritoneal papillary serous carcinoma,PPSC)的临床病理学特征、免疫表型、鉴别诊断及预后。方法对4例PPSC行免疫组化SP法染色及HPV检测,并复习相关文献。结果 4例PPSC均为女性,年龄23~62岁,平均51岁。临床表现多为无特异性的腹部不适,CA125升高,影像学表现为腹腔肿块。镜下肿瘤细胞形成形态不一、大小不等的乳头状结构;肿瘤细胞为低柱状,核质比大,核圆形,核仁大,异型性明显,核分裂象易见。腺体及间质内可见砂砾体。免疫表型:肿瘤细胞CA125、WT1、CK7、p53、CEA、p16均呈阳性,部分肿瘤细胞表达CK5/6、ER、PR;不表达GCDFP-15、CR、D2-40、CK20、Villin、CDX2、TTF1;HPV检测阴性。结论 PPSC是一种少见的原发于腹膜的恶性肿瘤,临床误诊率高,依据其临床和病理组织学特点,结合免疫组化染色可以明确诊断。     

涎腺导管癌5例临床病理分析

目的 探讨涎腺导管癌(salivary duct carcinoma,SDC)的临床病理特征、诊断和鉴别诊断、治疗及其预后.方法 回顾性分析5例 SDC 的临床资料、病理学形态和免疫组织化学标记结果.结果 (1)SDC好发于老年男性,平均发病年龄64.6岁,主要见于腮腺,生长迅速,易侵犯神经,常发生转移.(2)组织学特点与乳腺导管癌极为相似.肿瘤细胞向导管内生长,形成乳头状、筛孔状、实性等多种结构,并可形成粉刺样坏死,肿瘤向周围组织浸润生长是其显著特征.(3)免疫组化结果显示肿瘤细胞CK、EMA、CEA、GCDFP-15、c-erbB-2、p53均阳性,Ki-67增殖指数较高,ER、PR在部分病例呈散在阳性表达,而PSA、CD117、p63、S-100、CD10、GFAP均阴性.(4)随访资料显示SDC具有较差的预后.该组5例中,3例复发,1例转移.结论 SDC是一种高度恶性的涎腺肿瘤,组织形态类似乳腺导管癌.对该肿瘤确诊主要依赖于病理形态特征与免疫组化结果相结合,且需要和其他肿瘤如低度恶性筛状囊腺癌、黏液表皮样癌、实性腺样囊性癌、腺泡细胞癌、乳头状囊腺癌、多形性低度恶性腺癌、上皮-肌上皮癌等鉴别.     

子宫浆液性癌及内膜上皮内癌临床病理分析

目的探讨子宫浆液性癌(USC)及内膜上皮内癌(EIC)的组织病理学特点及鉴别诊断。方法回顾性分析13例USC和1例EIC的临床和病理学特点,并进行免疫组化染色观察p53、ER和PR在肿瘤细胞中的表达。结果患者年龄60～77岁(平均68岁),临床多表现为绝经后不规则阴道流血。随访10例,6例死亡,生存5～28个月(平均14个月);4例仍存活(11～36个月)。9例为单纯USC,4例混合有其他类型内膜腺癌成分。形态上,9例USC以有纤维血管轴心的宽乳头为主,被覆异型性明显的多角形或鞋钉样细胞,2例肿瘤大部分为纤细的乳头结构,1例以腺管状结构为主。2例表现为息肉表面的EIC,无间质浸润和腺体融合。3例USC的浸润成分以腺管结构为主。7例USC伴有EIC,其中2例宫颈内口、1例输卵管黏膜也可见上皮内癌改变。免疫表型:12例USC和所有EIC病变的非典型细胞对p53呈弥漫性强阳性反应,各有1例USC有ER、PR阳性表达。结论USC结构有一定的多样性,可以表现为分化好的腺管状结构。内膜活检标本中发现EIC改变时,提示可能有USC存在。应仔细检查绝经后妇女的内膜息肉以排除EIC改变。p53、ER、PR免疫组化染色有助于鉴别诊断。     

Thymic carcinoma: a clinicopathological and immunohistological study of 19 cases

Aims:  To study 19 cases of primary thymic carcinoma in order to define the clinicopathological features and the precise histochemical profile of this rare and heterogeneous group of tumours of the anterior mediastinum.
Methods and results:  The study group consisted of 13 males and six females, with a mean age of 58.5 years (range 29–75 years). Superior vena cava syndrome and chest pain were the main presenting symptoms. Three patients were asymptomatic. No patient had myasthenia gravis. Six different histological types were identified: neuroendocrine tumours (six patients), epidermoid carcinoma (five patients), sarcomatoid carcinoma (three patients), lymphoepithelioma-like carcinoma (two patients), mucoepidermoid carcinoma, clear cell carcinoma, and undifferentiated carcinoma (one patient each). The clear cell carcinoma was associated with a thymic cyst. No association with thymoma was observed. Surgical resection, performed in 10 cases, was complete in two. Sixteen patients received thoracic radiation, and 11 received systemic chemotherapy. Follow-up information was available in 16 cases; 12 patients presented with local or metastatic relapse, and 10 patients died of their tumour. The overall 5-year survival was 14.5%.
Conclusion:  Primary thymic carcinoma is a very heterogeneous group of tumours of the anterior mediastinum with an aggressive clinical behaviour, and a poor overall prognosis.     

Minimal deviation endometrioid adenocarcinoma of cervix: a clinicopathological and immunohistochemical study of two cases

We describe the clinicopathological features of two cases of minimal deviation endometrioid adenocarcinoma of cervix. This is a rare tumour whose predominant pattern is one of bland endometrial-type glands infiltrating the cervical wall without a stromal response. Thus, it may be confused with benign conditions such as cervical endometriosis. The two cases demonstrate that this tumour may behave aggressively despite its bland appearances. An immunohistochemical study was performed and showed only focal reactivity of neoplastic glands for carcinoembryonic antigen, which would limit its diagnostic use in small biopsy specimens.     

子宫乳头状浆液性癌临床病理分析及其辅助疗法探讨

目的 探讨子宫乳头状浆液性癌的临床病理特点及其合理疗法,以提高对该病的认识.方法 收集61例子宫乳头状浆液性癌,全面手术病理分期并随访4～9年,采用HE和免疫组织化学(EnVision法)染色,进行镜下观察,结合术后治疗方案和随访资料进行临床病理分析.结果 61例患者均为绝经后妇女,中位年龄68岁,临床表现为绝经后阴道流血和(或)腹部症状,或宫颈细胞学筛查发现异常等.肿瘤直径中位数7.5 cm(范围1.2～14.8 cm),FIGO分期:Ⅰ期17例(27.9%;Ⅰ A期8.2%,Ⅰ B期14.8%,Ⅰ C期4.9%),Ⅱ、Ⅲ和Ⅳ期分别占9.8%(6/61)、32.8%(20/61)和29.5%(18/61).活检和手术标本的组织学特点与卵巢高级别浆液性乳头状癌相似,以高级别核为特征,常出现复杂的分支状乳头状结构,沙砾体出现率24.6%(15/61),免疫组织化学染色示p53和Ki-67弥漫强阳性而雌激素受体(ER)和孕激素受体(PR)阴性(均为肿瘤细胞核着色).24.6%(15/61)未见子宫肌层浸润,但其中10/15有子宫外扩散,主要累及腹膜(6/15)和淋巴结转移(9/15).深肌层浸润、淋巴结转移和脉管受累为单个预后差的指标.56例接受术后辅助治疗,化疗者42例,放疗者24例,联合放/化疗10例.化疔组和未化疗组(用或不用放疗)的中位生存期分别为66.4和32.8个月.结论 子宫乳头状浆液性癌有独特的临床和病理特征,分期、淋巴结状况、脉管受累和肌层浸润深度为主要预后指标.晚期患者和复发患者采用含有紫衫醇(单用或联合使用顺铂)的全身化疗方案,可延长患者生存期.     

Papillary serous adenocarcinoma of the uterine cervix: a report of three cases.

Three cases of primary papillary serous adenocarcinoma of the uterine cervix, the first described in detail at this site, are reported. Two women, 32 and 33 years of age, presented with postcoital spotting. The third patient, a 69-year-old asymptomatic woman, had abnormal cells on a routine cervical Papanicolaou smear. There was no evidence of extracervical spread on physical examination in any of the cases, but computed tomographic scan of the abdomen revealed retroperitoneal lymphadenopathy in one patient. Two patients, one treated by preoperative radiation and cervicectomy and the other by radical hysterectomy and postoperative radiation, are alive and well 5 years later. The third patient underwent cone biopsy and is currently completing chemotherapy to be followed by radiation therapy. On gross examination, the tumors were indistinguishable from typical endocervical adenocarcinoma. Microscopic examination revealed high-grade papillary serous adenocarcinoma in each case; in one tumor, there was a minor admixed component of low-grade villoglandular papillary adenocarcinoma. Although none of the tumors was deeply invasive, pelvic nodal metastases were present in two cases. There was no evidence of endometrial, tubal, ovarian, or peritoneal tumor in any case. Papillary serous carcinomas should be distinguished from other papillary carcinomas of the cervix, particularly the recently described low-grade papillary villoglandular adenocarcinoma, a variant of endocervical adenocarcinoma with a good prognosis.     

Cytology of small-cell carcinoma of the uterine cervix in serous effusion: a report on two cases

Cytologic features of 2 cases of small-cell carcinoma of the uterine cervix in the body fluid are described. Case 1 was a 34-yr-old woman with a stage IIA cervical tumor. Pleural effusion developed 6 mo after initial diagnosis. Case 2 was a 38-yr-old woman with a stage IB tumor. Ascites was detected 11 mo after hysterectomy. Histologically, both cervical tumors were indistinguishable from small-cell carcinoma of oat-cell type in the lungs or other sites. Cytologically, the tumor cells in the pleural effusion of case 1 had characteristic features of small-cell carcinoma, including nuclear molding. However, almost all tumor cells in the ascites of case 2 showed a single-cell pattern mimicking malignant lymphoma. Mitotic figures and karyorrhetic bodies were occasionally seen. Nuclear molding was rarely identified. Small-cell carcinoma should be included in the differential diagnosis of malignant effusions containing lymphoma-like cells.     

Primary lymphoepithelioma-like carcinoma of salivary glands: a clinicopathological study of 21 cases

Lymphoepithelioma-like carcinoma (LELC) of salivary glands is a rare kind tumor. In this study, the authors evaluated 21 patients with LELC of salivary glands who had long-term follow-up. Clinical characteristics, Epstein-Barr virus (EBV) infection, immunohistochemical features, oncoprotein expression, treatments, and outcomes were analyzed. All patients were Chinese. Their ages ranged from 20 to 73 years. All tumors showed the typical syncytial growth pattern of undifferentiated epithelial cells with a significant lymphocyte reaction. All of patients were found by in situ hybridization to have the EBV genome. All tumors showed positive immunostaining of AE1/AE3, CK5/6 and p63. Nearly all cases had bcl-2 oncoprotein expression, but the detection rate of p53, and c-erb B-2 expression was extremely low. LELC of salivary glands is a distinct entity of salivary cancer. LELC of salivary glands can receive multimodality treatment and has a better prognosis similar to that of nasopharyngeal carcinoma.     

子宫内膜移行细胞癌临床病理学观察

目的观察子宫内膜移行细胞癌（transitional cell carcinoma of the endometrium,ETCC）的临床病理特点,探讨其病理诊断、组织发生、生物学行为及预后。方法采用光镜、免疫组化对3例ETCC进行病理观察,并复习相关文献。结果3例ETCC患者均表现为绝经后不规则阴道流血。大体上,肿瘤呈息肉状生长伴浅肌层浸润。镜下,肿瘤表现为乳头状结构,乳头表面覆盖多层异型上皮细胞,中央为纤维血管中心柱,其周围肿瘤细胞呈单层栅栏状排列,乳头表面细胞呈扁平状。乳头内可见腺样结构,部分乳头呈实性团状排列,中央见大片坏死。肿瘤细胞大小相对较一致,核呈圆形、卵圆形,核膜明显,染色质呈空泡状,未见核沟,核中度异型,核分裂象15～20／10HPF。浸润肌层的为分化差的巢状移行细胞成分。除了主要TCC成分外,2例分别混合有透明细胞癌及内膜样型腺癌。免疫组化染色结果示肿瘤细胞CK7＋、CK20-。结论ETCC是一种少见的子宫内膜癌的组织学亚型,形态学上具有尿路移行上皮分化特征,但保持了苗勒管的免疫表型CK7＋／CK20-。诊断ETCC的关键在于认识移行细胞的形态学特征。     

Microsatellite instability is uncommon in uterine serous carcinoma.

Thirty-four uterine serous carcinomas, a type of endometrial carcinoma with aggressive behavior and a high frequency (90%) of p53 gene mutations, were analyzed for microsatellite instability (MI). Genomic DNA isolated from paired normal and tumor tissue was analyzed at eight microsatellite loci (D2S119, D2S123, D2S147, D10S197, D13S175, D18S58, D18S69, and ATn) located on four different chromosomes. All 34 tumors failed to meet the criteria for MI, defined as an alteration in the size of at least two of the microsatellite loci in tumor DNA when compared with normal DNA. Only three tumors demonstrated a shift in the size of a single microsatellite locus. Previously we reported MI in 20% of uterine endometrioid carcinomas, the most common type of endometrial carcinoma. The observed difference in the MI frequency between endometrioid and serous carcinoma is statistically significant (P = 0.003). Our data demonstrate that MI is uncommon in uterine serous carcinoma and support that different pathogenetic mechanisms are involved in the development of the two most common types of endometrial carcinoma.     

Endocervical involvement by endometrial carcinoma on fractional curettage: a clinicopathological study of 37 cases.

This is a retrospective study of 37 patients with endometrial carcinoma and presence of tumor on endocervical curettage (clinical Stage II). We intended to correlate the presence or absence of endocervical stromal invasion with the clinical behavior and other prognostic factors. Based on the endocervical curettage, three categories (CAT) were defined: CAT I: tumor fragments only (seven cases); CAT II: endocervical tissue and free-floating tumor fragments (13 cases); and CAT III: endocervical tissue and tumor with evidence of stromal invasion (17 cases). Five tumors were partly of clear cell and/or papillary serous types and three of them belonged to CAT I. Six of seven tumors with a nuclear Grade 3 were in CAT III (p less than 0.05). Nine patients had local recurrence, metastases, or died of their disease (median follow-up: 56 mo) and seven of them were in the CAT III (p less than 0.05). We conclude that despite the presence of tumor on the endocervical curettage, the lack of endocervical tissue invasion is associated with a lower nuclear grade and a less aggressive behavior. These tumors should be regarded and treated as Stage I disease. Special attention must be paid to staging of clear cell and papillary serous adenocarcinomas because of the tendency for these tumors to contaminate the endocervical curettage.